ICD-10: C81.35

Lymphocyte Depleted Hodgkin Lymphoma (LDHL) is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. This specific type of lymphoma is known for its aggressive nature and is often associated with advanced disease at diagnosis. The ICD-10 code C81.35 specifically refers to cases of lymphocyte depleted Hodgkin lymphoma that are localized in the lymph nodes of the inguinal region and lower limb.

Clinical Description of Lymphocyte Depleted Hodgkin Lymphoma

Characteristics

• Histological Features: LDHL is marked by the presence of large Reed-Sternberg cells, which are the hallmark of Hodgkin lymphoma, alongside a notable reduction in the number of lymphocytes. This depletion can lead to a more pronounced inflammatory response and fibrosis within the lymphatic tissue[1].
• Symptoms: Patients may present with symptoms such as painless lymphadenopathy, fever, night sweats, weight loss, and pruritus. The inguinal region may exhibit swelling due to enlarged lymph nodes, which can cause discomfort or pain in the lower limbs[1][2].

Epidemiology

• Incidence: LDHL is less common than other subtypes of Hodgkin lymphoma, accounting for a small percentage of cases. It is more frequently diagnosed in older adults and is often associated with a poorer prognosis compared to other forms of Hodgkin lymphoma[2][3].
• Risk Factors: Factors that may increase the risk of developing LDHL include a history of Epstein-Barr virus infection, immunosuppression, and certain genetic predispositions[3].

Diagnosis and Staging

• Diagnostic Procedures: Diagnosis typically involves a combination of physical examination, imaging studies (such as CT scans), and biopsy of affected lymph nodes. Histopathological examination is crucial for confirming the diagnosis of LDHL[1][2].
• Staging: The Ann Arbor staging system is commonly used to determine the extent of the disease. LDHL in the inguinal region and lower limb would likely be classified as stage II or higher, depending on the involvement of other lymphatic regions or systemic symptoms[2].

Treatment Options

• Chemotherapy: The primary treatment for LDHL often involves chemotherapy regimens, which may include combinations of agents such as doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) or other protocols tailored to the patient's specific condition[3].
• Radiation Therapy: In some cases, radiation therapy may be utilized, particularly if the disease is localized and has not spread extensively[1].
• Stem Cell Transplantation: For patients with relapsed or refractory disease, autologous stem cell transplantation may be considered as a treatment option[2].

Prognosis

The prognosis for patients with lymphocyte depleted Hodgkin lymphoma can be variable, often depending on the stage at diagnosis and the patient's overall health. Generally, LDHL is associated with a poorer prognosis compared to other subtypes of Hodgkin lymphoma, necessitating aggressive treatment approaches[3].

In summary, ICD-10 code C81.35 pertains to lymphocyte depleted Hodgkin lymphoma localized in the inguinal region and lower limb, characterized by specific clinical features, diagnostic criteria, and treatment protocols. Understanding these details is crucial for effective management and improved patient outcomes.

References

1. Article - Billing and Coding: Allogeneic Hematopoietic Cell Transplantation for Hodgkin Lymphoma.
2. Hodgkin lymphoma C81 - ICD-10-CM Codes.
3. Medical policy on hematopoietic cell transplantation for Hodgkin lymphoma.

Lymphocyte depleted Hodgkin lymphoma (LDHL) is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes. The ICD-10 code C81.35 specifically refers to cases where this type of lymphoma is located in the lymph nodes of the inguinal region and lower limb. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for diagnosis and management.

Clinical Presentation

Signs and Symptoms

Patients with lymphocyte depleted Hodgkin lymphoma may present with a variety of signs and symptoms, which can include:

• Lymphadenopathy: The most common presentation is the enlargement of lymph nodes, particularly in the inguinal region. Patients may notice swelling or lumps in the groin area.
• B Symptoms: These systemic symptoms include:
• Fever: Often low-grade but can be persistent.
• Night Sweats: Profuse sweating during the night that can soak clothing and bedding.
• Weight Loss: Unintentional weight loss of more than 10% of body weight over six months.
• Fatigue: A general feeling of tiredness or lack of energy that is not relieved by rest.
• Pruritus: Itching of the skin, which can be generalized or localized.
• Pain: Some patients may experience pain in the affected lymph nodes, especially if they are compressing surrounding structures.

Patient Characteristics

Certain demographic and clinical characteristics are often associated with lymphocyte depleted Hodgkin lymphoma:

• Age: LDHL typically occurs in older adults, with a median age of diagnosis in the 30s to 50s. However, it can also present in younger individuals.
• Gender: There is a slight male predominance in cases of Hodgkin lymphoma, including the lymphocyte depleted subtype.
• Comorbidities: Patients may have a history of immunosuppression or other underlying health conditions, which can influence the presentation and progression of the disease.
• Histological Features: The diagnosis is confirmed through biopsy, revealing a predominance of Reed-Sternberg cells and a marked depletion of lymphocytes, which is characteristic of this subtype.

Diagnosis and Management

Diagnosis typically involves imaging studies such as CT scans to assess the extent of lymphadenopathy and possible involvement of other organs. A biopsy of the affected lymph nodes is essential for histological confirmation.

Management may include:

• Chemotherapy: Often the first line of treatment, with regimens tailored to the individual patient based on disease stage and overall health.
• Radiation Therapy: May be used in conjunction with chemotherapy, particularly for localized disease.
• Supportive Care: Addressing symptoms such as pain, fatigue, and nutritional needs is crucial for improving quality of life.

Conclusion

Lymphocyte depleted Hodgkin lymphoma, particularly when affecting the inguinal region and lower limb, presents with distinct clinical features and patient characteristics. Early recognition of symptoms such as lymphadenopathy, B symptoms, and fatigue is vital for timely diagnosis and treatment. Given its rarity and the complexity of management, a multidisciplinary approach involving oncologists, radiologists, and supportive care teams is often necessary to optimize patient outcomes.

ICD-10 code C81.35 refers specifically to "Lymphocyte depleted Hodgkin lymphoma, lymph nodes of inguinal region and lower limb." This classification is part of the broader category of Hodgkin lymphoma, which is a type of cancer that originates in the lymphatic system. Below are alternative names and related terms associated with this specific diagnosis.

Alternative Names for C81.35

1. Lymphocyte Depleted Hodgkin Lymphoma (LDHL): This is the full name of the condition, emphasizing the specific subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes.

2. Hodgkin's Disease, Lymphocyte Depleted Type: This term is often used interchangeably with lymphocyte depleted Hodgkin lymphoma, highlighting its classification as a type of Hodgkin's disease.

3. Hodgkin Lymphoma, Lymphocyte Depletion Variant: This name underscores the variant nature of the lymphoma, indicating its unique histological features.

Related Terms

1. Hodgkin Lymphoma: A broader term that encompasses all types of Hodgkin lymphoma, including lymphocyte depleted, mixed cellularity, and nodular sclerosis types.

2. Non-Hodgkin Lymphoma: While distinct from Hodgkin lymphoma, this term is often mentioned in discussions about lymphomas in general, as it represents a different category of lymphatic cancers.

3. Reed-Sternberg Cells: These are the abnormal cells found in Hodgkin lymphoma, including the lymphocyte depleted subtype, and are a key diagnostic feature.

4. Lymphadenopathy: This term refers to the enlargement of lymph nodes, which is a common symptom in patients with Hodgkin lymphoma, including those with C81.35.

5. Inguinal Lymphadenopathy: Specifically refers to the enlargement of lymph nodes in the inguinal region, which is relevant for the C81.35 diagnosis.

6. Lower Limb Lymphadenopathy: This term describes lymph node involvement in the lower limbs, pertinent to the specific location indicated by the C81.35 code.

Conclusion

Understanding the alternative names and related terms for ICD-10 code C81.35 is essential for accurate diagnosis, treatment planning, and communication among healthcare professionals. This knowledge aids in the identification of the specific subtype of Hodgkin lymphoma and its clinical implications, ensuring that patients receive appropriate care tailored to their condition.

Lymphocyte depleted Hodgkin lymphoma (LDHL) is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a scarcity of lymphocytes. The diagnosis of LDHL, particularly when it involves the lymph nodes of the inguinal region and lower limb, follows specific clinical, histopathological, and imaging criteria. Below is a detailed overview of the criteria used for diagnosis, particularly in relation to the ICD-10 code C81.35.

Clinical Presentation

1. Symptoms: Patients may present with:
   - Painless lymphadenopathy, particularly in the inguinal region.
   - Systemic symptoms such as fever, night sweats, and weight loss (often referred to as "B symptoms").
   - Fatigue and pruritus (itching) may also be reported.

2. Physical Examination:
   - Examination may reveal enlarged lymph nodes in the inguinal region and potentially in other areas, such as the axillary or cervical regions.

Histopathological Criteria

1. Biopsy: A definitive diagnosis of LDHL requires a lymph node biopsy. The histological examination typically reveals:
   - A predominance of Reed-Sternberg cells.
   - A marked depletion of lymphocytes, which is a distinguishing feature of this subtype.
   - The presence of a fibrotic background and inflammatory cells, which may include eosinophils and plasma cells.

2. Immunohistochemistry:
   - Reed-Sternberg cells are typically positive for CD30 and CD15, which are important markers in confirming the diagnosis.
   - Additional markers such as PAX5 and MUM1 may also be assessed to further characterize the tumor.

Imaging Studies

1. CT or PET Scans: Imaging studies are crucial for staging and assessing the extent of the disease. These may include:
   - CT scans of the chest, abdomen, and pelvis to identify lymphadenopathy and any organ involvement.
   - PET scans can help in evaluating metabolic activity and detecting any additional sites of disease.

2. MRI: In some cases, MRI may be used to assess soft tissue involvement, particularly in the lower limbs.

Staging

The Ann Arbor staging system is commonly used for Hodgkin lymphoma, which includes:
- Stage I: Involvement of a single lymph node region.
- Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm.
- Stage III: Involvement of lymph node regions on both sides of the diaphragm.
- Stage IV: Disseminated involvement of one or more extralymphatic organs.

For LDHL specifically affecting the inguinal region and lower limb, the staging would depend on the extent of lymph node involvement and any systemic symptoms present.

Laboratory Tests

1. Blood Tests: Routine blood tests may show:
   - Anemia or thrombocytopenia, which can occur due to bone marrow involvement.
   - Elevated lactate dehydrogenase (LDH) levels, which may indicate a more aggressive disease.

2. Bone Marrow Biopsy: This may be performed to assess for bone marrow involvement, which can influence treatment decisions.

Conclusion

The diagnosis of lymphocyte depleted Hodgkin lymphoma, particularly in the context of the inguinal region and lower limb, involves a comprehensive approach that includes clinical evaluation, histopathological confirmation through biopsy, imaging studies for staging, and laboratory tests to assess overall health and disease extent. Accurate diagnosis is crucial for determining the appropriate treatment strategy and improving patient outcomes. If you have further questions or need additional information, feel free to ask!

Lymphocyte depleted Hodgkin lymphoma (LDHL), classified under ICD-10 code C81.35, is a rare subtype of Hodgkin lymphoma characterized by a predominance of Reed-Sternberg cells and a significant depletion of lymphocytes. This subtype often presents with advanced disease and is associated with a poorer prognosis compared to other forms of Hodgkin lymphoma. The treatment approaches for LDHL typically involve a combination of chemotherapy, radiation therapy, and, in some cases, stem cell transplantation. Below is a detailed overview of the standard treatment modalities for this condition.

Standard Treatment Approaches

1. Chemotherapy

Chemotherapy is the cornerstone of treatment for lymphocyte depleted Hodgkin lymphoma. The most commonly used regimens include:

• ABVD Regimen: This includes Adriamycin (doxorubicin), Bleomycin, Vinblastine, and Dacarbazine. While ABVD is the standard treatment for many Hodgkin lymphoma subtypes, its effectiveness in LDHL may be limited due to the aggressive nature of this variant.

• BEACOPP Regimen: This regimen (Bleomycin, Etoposide, Adriamycin, Cyclophosphamide, Oncovin, Procarbazine, and Prednisone) is often considered for patients with advanced disease or those who do not respond adequately to ABVD. BEACOPP has shown improved outcomes in some studies for patients with more aggressive forms of Hodgkin lymphoma, including LDHL.

• Escalated BEACOPP: For patients with high-risk features, escalated BEACOPP may be employed, which involves higher doses of the same drugs used in the standard BEACOPP regimen.

2. Radiation Therapy

Radiation therapy may be utilized in conjunction with chemotherapy, particularly for localized disease or as consolidation therapy after chemotherapy. The use of radiation is tailored based on the extent of the disease and the response to initial treatment.

• Involved Field Radiation Therapy (IFRT): This technique targets only the areas affected by lymphoma, minimizing exposure to surrounding healthy tissues.

• Total Body Irradiation (TBI): In cases where stem cell transplantation is considered, TBI may be used as part of the conditioning regimen.

3. Stem Cell Transplantation

For patients with relapsed or refractory lymphocyte depleted Hodgkin lymphoma, autologous stem cell transplantation (ASCT) may be considered. This approach involves harvesting the patient's own stem cells, administering high-dose chemotherapy to eradicate the lymphoma, and then reinfusing the stem cells to restore bone marrow function.

• Allogeneic Stem Cell Transplantation: In certain cases, allogeneic transplantation from a matched donor may be an option, particularly for patients with poor prognostic factors or those who have not responded to other treatments.

4. Targeted Therapy and Immunotherapy

While traditional chemotherapy remains the primary treatment, there is ongoing research into targeted therapies and immunotherapies for Hodgkin lymphoma. Agents such as brentuximab vedotin (Adcetris®), an antibody-drug conjugate targeting CD30, have shown promise in treating relapsed or refractory cases of Hodgkin lymphoma, including LDHL.

5. Clinical Trials

Given the rarity of lymphocyte depleted Hodgkin lymphoma, participation in clinical trials may be a viable option for patients. These trials often explore new treatment combinations, novel agents, and innovative approaches to improve outcomes.

Conclusion

The management of lymphocyte depleted Hodgkin lymphoma requires a multidisciplinary approach tailored to the individual patient's disease characteristics and overall health. Standard treatment typically involves a combination of chemotherapy, radiation therapy, and potentially stem cell transplantation, with ongoing research into targeted therapies and immunotherapy. Given the complexity and rarity of this subtype, consultation with a specialist in hematologic malignancies is crucial for optimal treatment planning and management.